Neuroblastoma incidence and survival in European children (1978-1997): Report from the Automated Childhood Cancer Information System project.

Abstract The Automated Childhood Cancer Information System (ACCIS) collects and presents data on childhood cancer in Europe. This report describes trends (1978-1997) and geographical differences (1988-1997) in incidence and survival for 6202 children with neuroblastoma from 59 registries in 19 countries, grouped into five regions (British Isles, West, East, North, and South). The age-standardised incidence rate (ASR) of neuroblastoma in Europe in 1988-1997 was 10.9 cases per million children, being highest in infants (52.6). The ASR of neuroblastoma increased in Europe from 8.4 in 1978-1982 to 11.6 in 1993-1997, mostly due to an increase in infants (from 35.4 to 57.8). Overall 5-year survival was 59%, ranging from 47% (East) to 67% (West). It improved markedly from 37% in 1978-1982 to 66% in 1993-1997, especially in infants. A certain amount of overdiagnosis in children under 2 years of age may explain the increased incidence rates and partially the increase in survival. Survival of older children (aged 2-14 years), which is likely to be largely affected by therapy, has also improved from 21% to 45%.